Phenylketonuria is a rare inherited metabolic defect in which there is a deficiency of the liver enzyme phenylalanine hydroxylase. Unless this condition is diagnosed within the first few weeks of life, and treated with special diets which have a high carbohydrate content, but which are very low in phenylalanine, severe mental deficiency can result. 105 phenylketonuric (PKU) children, aged 10 months to 16 years, were examined to determine whether this special diet had any effect on the prevalence of caries in PKU children. It was concluded that caries experience in the PKU children examined was of the same order as that reported in normal children of comparable age.

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